SAPHO syndrome
SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) is a rare, often-overlooked member of the spondyloarthropathy spectrum defined by sterile inflammatory bone disease, classically anterior chest wall hyperostosis, occurring together with severe acne or palmoplantar pustulosis. It is a clinical diagnosis supported by the technetium bone scan's characteristic bull's head uptake pattern, with a sterile, culture-negative bone biopsy the key step that excludes its most important mimic, infectious osteomyelitis.
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What this article covers
- Overview
A bone-skin syndrome named for its own acronym: SAPHO stands for Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis, describing aseptic neutrophilic skin disease occurring with aseptic (sterile) osteoarticular disease.
- Epidemiology
Bimodal age distribution: SAPHO affects children (overlapping with chronic recurrent multifocal osteomyelitis, its pediatric-onset counterpart, where osteitis predominates without prominent skin disease) and young to middle-aged adults.
- Risk factors
Cigarette smoking: the strongest modifiable association, acting through its link to palmoplantar pustulosis.
- Pathophysiology
Sterile, not infectious, bone inflammation: the process behaves as an autoinflammatory bone-skin disorder with dysregulated innate immune/cytokine signaling rather than true infection, alongside…
- Clinical features
Anterior chest wall pain, the hallmark and most common presentation: insidious or subacute pain, tenderness, and sometimes swelling over the sternoclavicular, sternocostal, or manubriosternal joints…
- Differential diagnosis
Osteomyelitis: the most dangerous mimic, always excluded first, producing the same sclerotic, hyperostotic bone appearance on radiograph and bone scan; fever and leukocytosis are inconsistent (often…
- Diagnosis
How the diagnosis is established: SAPHO is a clinical diagnosis, made by recognizing skin disease (severe acne or palmoplantar pustulosis, or a related follicular occlusion/pustular disorder)…
- Management
First, exclude and treat infection or malignancy if identified: a positive biopsy culture or true sepsis is treated as osteomyelitis with organism-directed antibiotics for at least 6 weeks, not…
- Complications
Missed osteomyelitis: attributing a positive-culture infection to SAPHO and withholding antibiotics is the most serious error, given the identical imaging appearance.
- Prognosis
A chronic, relapsing-remitting course: SAPHO typically follows years of exacerbations and remissions rather than a single self-limited illness; skin and bone activity do not always flare together.
- Prevention
Smoking cessation: offer pharmacotherapy at every visit, since smoking is strongly linked to palmoplantar pustulosis flares; varenicline 0.5 mg PO daily for days 1-3, then 0.5 mg PO twice daily for…
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